Volume 20, Issue 80 (1-2012)                   JGUMS 2012, 20(80): 92-98 | Back to browse issues page

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Azizi Asl M, Koohmanaei S, Darbandi B, Mirbazegh S. Bilateral Pheochromocytomain a Child with Atypical Primary Presentation A Very Rare Case. JGUMS 2012; 20 (80) :92-98
URL: http://journal.gums.ac.ir/article-1-124-en.html
1- Gilan University of Medical Sciences
2- Gilan University of Medical Sciences , koohmana@yahoo.com:
3- Tehran University of Medical Sciences
Abstract:   (10035 Views)
Abstract Introduction: Pheochromocytoma is a rare cathecolamine-producing tumor that arises from chromaffin cells of the adrenal medulla or extra-adrenal paraganglionic tissues, and has different and variant presentations. Only 10% of pheochromocytomas occur in children with an incidence of approximately 1 case per 100,000 patient-years. It may be completely asymptomatic, or present with symptoms and signs such as headache, dizziness, palpitation, diaphoresis or hyperhydrosis, and hypertension. Bilateral pheochromocytoma - specially in children-is very rare and almost familial, but it has few sporadic cases. The diagnosis of isolated bilateral pheochromocytoma depends on ruling out of congenital etiologies such as type 2 Multiple Endocrine Neoplasia (MENII), Von Hippel Lindau disease (VHL), type 1 Neurofibromatosis (NF I), Tuberous Sclerosis (TS), and Sturge Weber. Case Report: The patient was a 12 year old boy who was referred with unusual suspicious symptoms of pheochromocytoma (weakness, dizziness, and hyperhydrosis) since the last year. During the disease, he had episodic headaches without hypertension. He had not any problem or disease until one year before. Hematologic exam revealed an elevated Erythrocyte Sedimentation Rate. In renal sonography and scan, we had hydonephrosis and pyelocaliceal dilatation.The collected 24hour urine contained elevated load of cathecolamine methabolites. Abdominal CT showed solid enhanced tumors in both adrenals, confirmed by MIBG scan. The patient was treated with bilateral adrenalectomy and diagnosis was confirmed by pathology and IHC staining. Conclusion: Although most pheochromocytomas have typical presentations, they may present with unusual symptoms and signs like the present case. So we should consider pheochromocytoma in patients with familial history of the disease, patients with typical symptoms or hypertension, and also patients with unusual presentations.
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Review Paper: Research | Subject: General
Received: 2013/11/6 | Accepted: 2014/04/13 | Published: 2014/04/13

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